Catdiomyopathies are diseases that primarily affect the myocardium,leading to serious cardiac dysfimction and heart failure.Out of the three major categories of candiomyopathies(hypertrophic,dilated and restrictive),restrictive cardiomyopathy(RCM)is less common and also the least studied However,the prognosis for RCM is poor as some patients dying in their childhood The molecular mechanisms behind the disease development and progression are not very clear and the treatment of RCM is very difficult and often ineffective.In this article,we reviewed the recent progress in RCM research from the clinical studies and the translational studies done on diseased transgenic animal models.This will help for a better understanding of tare mechanisms underlying the etiology and development of RCM and for the design of better treatments for the disease.

Purpose: Hepatocellular carcinoma (HCC) patients with major portal vein tumor thrombosis (mPVTT) complications were generally characterized by extremely poor prognoses. The aim of this study was to explore the role of 18F-fluorodeoxyglucose (18F-FDG) PET/CT imaging in predicting HCC complicated by mPVTT. Methods: Five hundred one HCC patients received surgery in our hospital during November 2008 to December 2014, among which 32 patients (6.4%) were diagnosed as HCC complicated by mPVTT. Six cases were excluded for reasons of complex medical conditions, including 2 cases of salvage liver transplantation, 2 cases of re-resection, 1 case of mPVTT combined with inferior vina cava tumor thrombosis, and 1 case of residual portal vein tumor thrombosis. Ultimately, 26 cases were enrolled in this study. The maximal tumor standardized uptake value (SUVmax) was identified as a predictive factor and detected. The univariate and multivariate regression analyses were performed to identify the prognostic factors for recurrence-free survival (RFS) and overall survival (OS) of HCC patients complicated by mPVTT. Results: Our results showed that the median OS was 16 months. The 1-, 3-, and 5-year cumulative OS was 55.6%, 31.7%, and 31.7%, respectively. The multivariate regression analysis revealed that SUVmax ≥ 4.65 was the only independent risk factor for RFS and OS. Conclusion SUVmax was an independent predictor for RFS and OS of patients suffering from both HCC and mPVTT. L ow SUVmax could serve as an effective factor for selecting candidates with low recurrence risks and for helping with improving patient survival after surgical resection.

PURPOSE: Tamoxifen has been well known as an effective anti-tumor agent against breast cancer. The important role of bcl-2 and p53 proteins in tamoxifen-induced apoptosis of breast cancer cells has been suggested. However, the paradoxical fact that bcl-2 over-expression is assdegrees Ciated with better prognosis in clinic has not yet been clearly explained. To investigate this paradox, we analyzed the effect and dynamics of bcl-2 and p53 on the apoptosis after treatment of breast cancer cells with tamoxifen. MATERIALS AND METHODS: The human breast cancer cell lines MCF-7 and MB MDA-468 were treated with 17-betaestradiol (E2) and tamoxifen. RESULTS: Following tamoxifen treatment, MCF-7 cells underwent apoptosis accompanied by reduced bcl-2 expression. E2 pre-treatment led to the inhibition of tamoxifen-mediated apoptosis and bcl-2 down-regulation. When MB MDA-468 cells were treated with E2 or tamoxifen, bcl-2 and p53 protein expression did not change and apoptosis did not develop. CONCLUSION: We observed that the down-regulation of bcl-2 by tamoxifen treatment can facilitate the apoptosis of breast cancer cells without p53 mutations. This finding was consistent with clinical experiences in which bcl-2 positive tumors were assdegrees Ciated with more indolent phenotypes in breast cancer.
Apoptosis* ; Breast Neoplasms* ; Breast* ; Cell Line* ; Down-Regulation ; Humans* ; MCF-7 Cells ; Phenotype ; Prognosis ; Tamoxifen

Hereditary spastic paraplegia is a familial disorder which is inherited by autosomal dominant, autosomal recessive or sex linked pattern. We experienced a family who has hereditary spastic paraplegia with mental retardation and extrapyramidal symptom that is thought inherited by autosomal dominant inheritance pattern. A review of literatures was made briefly.
Humans ; Inheritance Patterns ; Intellectual Disability ; Spastic Paraplegia, Hereditary*

Hereditary spastic paraplegia is a familial disorder which is inherited by autosomal dominant, autosomal recessive or sex linked pattern. We experienced a family who has hereditary spastic paraplegia with mental retardation and extrapyramidal symptom that is thought inherited by autosomal dominant inheritance pattern. A review of literatures was made briefly.
Humans ; Inheritance Patterns ; Intellectual Disability ; Spastic Paraplegia, Hereditary*

OBJECTIVETo investigate the anti-fibrotic effects of Qidan granule in rats.

METHODSThe rats were randomly divided into six experimental groups: normal group, model group, Qidan group, Tetrandrine group. All rats except normal group were treated with silicon dioxide (50 mg/rat) by intratracheal instillation to induce silicosis. Qidan group and Tetrandrine group were treated with Qidan granule (3125 mg/kg) or treated with Tetrandrine (22 mg/kg) respectively. All the rats were sacrificed after 5 months. Calculate Lung/body coefficient by weighting the lung wet weight and the body weight of rats. Content of Hydroxyproline was measured by alkaline hydrolysis. The gene expression of transforming growth factor-beta1 was examined by using enzyme-linked immunosorbent assay (ELISA). Paraffin embedded lung sections with HE staining, VG staining and Gomori staining were observed under light microscope.

RESULTSIn Qidan group and Tetrandrine group, Lung/body coefficient and content of Hydroxyproline and expression of transforming growth factor-beta1 were lower as compared with model group (P < 0.05). Model group mainly showed III approximately IV grade silicotic nodule, which contained thick collagen and sparse reticulum fibe; Qidan group and Tetrandrine group appeared with II grade silicotic nodule, which contained tiny collagen and intensive reticulum fibe. Tetrandrine group showed injury of kidney, and others were normal.

CONCLUSIONQidan granule extract should prevent and from inhibit the remarkably silicotic fibrosis in rats.

Animals ; Disease Models, Animal ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Phytotherapy ; Pulmonary Fibrosis ; pathology ; prevention & control ; Rats ; Rats, Wistar ; Silicosis ; drug therapy ; metabolism ; pathology ; Transforming Growth Factor beta ; biosynthesis

OBJECTIVETo observe the difference in the efficacy on the symptoms of bronchial asthma at the chronic persistent stage between acupoint heat-sensitive moxibustion and western medicine with Seretide.

METHODSSixty-four cases were randomly divided into a heat-sensitive moxibustion group (32 cases) and a western medication group (32 cases). In the heat-sensitive moxibustion group, the sensitized points located between Feishu (BL 13) and Geshu (BL 17) or in the region 6-cun lateral from the 1st and the 2nd intercostal spaces of the chest were selected. The heat-sensitive moxibustion was adopted, continuously for 8 days, once per day. In the later 22 days of the 1st month, 12 treatments should be ensured. Two months later, 15 treatments should be guaranteed each month. The time of each treatment was 30 to 90 min. Totally 50 treatments were required. In the western medication group, Seretide inhaler was adopted, one inhalation each time, twice per day, for 3 months totally. The asthmatic symptoms were scored for the patients in two groups and the comparison was made between the two groups.

RESULTSAfter 3 months of treatment, the asthmatic symptom scores were all improved for the patients in the heat-sensitive moxibustion group and the western medication group as compared with those before treatment (both P < 0.05). In 6 months of follow-up visit, the asthmatic symptom scores in the heat-sensitive moxibustion group were stable, but those in the western medication group were reduced, there was significant difference between the two groups (P < 0.05).

CONCLUSIONThe acupoint heat-sensitive moxibustion effectively relieves the clinical symptoms for the patients with bronchial asthma at the chronic persistent stage. Its efficacy is similar to that of Seretide inhaler. But the long-term efficacy of the heat-sensitive moxibustion is much better.

Acupuncture Points ; Adolescent ; Adult ; Albuterol ; analogs & derivatives ; therapeutic use ; Androstadienes ; therapeutic use ; Asthma ; therapy ; Chronic Disease ; Drug Combinations ; Female ; Fluticasone-Salmeterol Drug Combination ; Humans ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Moxibustion ; methods

OBJECTIVETo investigate K(ATP) channel function of cardiomyocytes isolated from the left ventricular wall of rats with or without abdominal aortic constriction at different time points under normal or simulated ischemic conditions.

METHODSMale Wistar rats were randomized into 4 groups (n = 10 - 13): 4-week sham-operated group (F4), 4-week aortic-banded group (T4), 12-week sham-operated group (F12), 12-week aortic-banded group (T12). Chronic pressure overload model was established by abdominal aortic constriction. Left ventricular myocytes were isolated by modified Langendorff perfusion method post in vivo hemodynamical measurements. The whole-cell patch-clamp technique was used to record transient outward current of K(ATP) channel on myocytes under normal and simulated ischemic perfusion conditions. The current densities of K(ATP) channel between F4 and T4 group, F12 and T12 group were compared under 0 mV of test potential.

RESULTSSBP, DBP and MBP were significantly increased in T4 group compared to F4 group, but were similar between T12 and F12 groups. LVEDP and +/- dp/dtmax were similar between T4 and F4 groups and LVEDP was significantly increased while +/- dp/dtmax significantly reduced in T12 group than that in F12 group. Whole-cell membrane current densities were similar between F4 and T4 group or F12 and T12 group under normoxic condition, the K(ATP) current densities increased dramatically in T12 group [(28.11 +/- 3.91) pA/pF vs (11.55 +/- 1.17) pA/pF, P < 0.01], but not in T4 group [(14.09 +/- 5.74) pA/pF vs (11.74 +/- 3.68) pA/pF, P > 0.05] in myocytes exposed to ischemic solution for 25 minutes. The total number of K(ATP) channel in ventricular myocytes was similar between F4 and T4 group or F12 and T12 group.

CONCLUSIONSThe sarcolemmal K(ATP) channel was more sensitive to ischemia and the current magnitude was significantly increased at the stage of congestive heart failure. The functional change of K(ATP) channel occurred before the increase of total number of K(ATP) channel.

Animals ; Disease Progression ; Heart Failure ; metabolism ; physiopathology ; KATP Channels ; metabolism ; Male ; Myocytes, Cardiac ; pathology ; Patch-Clamp Techniques ; Rats ; Rats, Wistar

OBJECTIVETo discuss the treatment of the scrotal vein malformation in teenagers and clinical efficacy.

METHODS32 cases with the local and diffuse scrotal vein malformation were retrospectively analyzed. 31 cases underwent local injection with 40% urea before resection. The urea was injected locally into tumor through multi-points within 30 seconds, 2-6 ml every time, one time a day. The injection was performed for 5-12 days. The treatment was refused in one case. The therapeutic effect and cosmetic result were recorded.

RESULTSThe tumors were removed radically in 28 cases including one operation in 25 cases and secondary operation in 3 cases. The patients were followed up for 1-3 years with no recurrence. Cosmetic result with bilaterally symmetric scrotum was satisfactory. The tumors in 3 severe cases were partially resected with improvement.

CONCLUSIONSCombined treatment with urea injection and surgical procedure can effectively treat the scrotal vein malformation with satisfactory result.

Adolescent ; Humans ; Injections, Intralesional ; Male ; Retrospective Studies ; Scrotum ; blood supply ; Urea ; administration & dosage ; therapeutic use ; Vascular Malformations ; therapy ; Young Adult

Objective: To know the pre-treatment drug resistance ( PDR ) status of newly reported human immunodeficiency virus type 1 ( HIV-1 ) infected individuals in Wenzhou, so as to provide guidance for antiretroviral therapy ( ART ). Methods: Totally 232 plasma samples of newly reported HIV-1 infected individuals who had not received ART were collected in Wenzhou in 2019. Virus ( HIV-1 ) RNA was extracted, followed by reverse transcription PCR and nested PCR to amplify the pol region and sequence. Resistance mutations and resistance to non-nucleoside reverse transcriptase inhibitors ( NNRTIs ), nucleoside reverse transcriptase inhibitors ( NRTIs ) and protease inhibitors ( PIs ) was analyzed. Results: The pol region sequences from 199 infected patients were obtained and the incidence of PDR was 8.04% ( 16/199 ). Eight genotypes were detected, including circulating recombinant forms ( CRFs ) CRF07_BC ( 47.24%, 94/199 ) and CRF01_AE ( 29.15%, 58/199 ) which were the dominant types. Two unique recombinant forms ( URFs ) were detected, namely URF( CRF01_AE/BC ) and URF( B/C ) . Thirty-one cases ( 15.58% 31/199 ) had drug-resistant mutations. For NNRTIs, NRTIs and PIs, 20 cases ( 64.52% ) , 2 cases ( 6.45% ) and 9 cases ( 29.03% ) with drug resistance mutations were detected, respectively. The resistance mutations to NNRTIs included K101E, K103N/R, V106I, E138K, V179D/E/T, Y181C, G190A and H221Y. Four cases each had two resistance mutations to NNRTIs. The resistance mutations to NRTIs were V75M and M184V. The resistance mutations to PIs were M46I, L33F and Q58E. For the newly released NNRTI drug Doravirine ( DOR ), two cases were found to have mutations of resistance. Conclusions The incidence of PDR among newly reported HIV-1 patients in Wenzhou is 8.04%, mainly caused by NNRTIs drug-resistant mutation. Resistance to the new drug DOR has emerged. The surveillance of drug resistance should continue to be strengthened.